PEDs Exam 2

Daily Fluid Requirements

1-10 kg: - 100 mL/kg 11-20 kg: - 1000 mL puls 50 mL/kg for each kg >10 kg >20 kg: - 1500 mL plus 20 mL/kg for each kg >20 kg THEN: - Divide by 24 hours - 1-2 mL/kg/hr (normal child urine output)

Ductus Arteriosus

A blood vessel in a fetus that bypasses pulmonary circulation by connecting the pulmonary artery directly to the ascending aorta

Anemia

A condition in which the blood is deficient in red blood cells, in hemoglobin, or in total volume; concentration below normal Consequences: - Murmur production - Increased cardiac workload can lead to cardiac failure - Decreased oxygen-carrying capacity of blood - Decreased amount of oxygen available to tissues (Cyanosis) - Growth retardation, anorexia (chronic severe), delayed sexual maturation Diagnosis: - History & physical exam - CBC, Hgb, Hct - Sometimes defined as a Hgb <10 or 11 g/dL; however, this cutoff may be inappropriate for children Clinical Manifestations: - May have a murmur - Cyanosis - Growth retardation - Pica: Consumption of nonnutritive substances such as dirt, starch, paint-chips, paper Therapeutic/Nursing Management: - Reverse the anemia by treating the underlying cause - Transfusion after hemorrhage if needed - Nutritional intervention for deficiency anemias (Iron Supplements) - Supportive care - IV fluids - Oxygen - Bedrest - Ask about history of frequent milk drinking (iron-deficiency)

Aplastic Crisis

A condition in which the body stops producing red blood cells; typically caused by infection - Type of sickle-cell crisis

Shock

A condition in which the circulatory system fails to provide sufficient circulation to enable every body part to perform its function; also called hypoperfusion Types: - Hypovolemic - Distributive (Anaphylactic, Septic, Neurogenic) - Cardiogenic Clinical Manifestations: - Tachycardia & Tachypnea - Initially BP increases then decreases later on - Weak peripheral pulses - Initially skin is warm and then as it progressives gets cold and clammy Phases of Shock: - Compensated - Decompensated - Irreversible or Terminal Shock Therapeutic Management: - Ventilatory support - Fluid administration (20 mL/kg, Isotonic solutions 0.9% NS, LR) - Improve cardiac contractility (Dopamine, Epi) for Cardiogenic - Septic shock you need Antibiotics Nursing Care Management: - Monitor Vital Signs - Thorough heart & respiratory assessments - Monitor I & O's

Tetralogy of Fallot (TOF)

A congenital malformation of the heart involving four distinct defects - RIGHT TO LEFT SHUNTING, Cyanotic - Defects with decreased pulmonary blood flow 1.) Ventricular septal defect (VSD) 2.) Pulmonic stenosis 3.) Overriding Aorta 4.) Right ventricular hypertrophy Clinical Manifestations: - Cyanotic after birth - Mild cyanosis that progresses over the first year of life - Pulmonary stenosis worsens - Murmur - Blue spells/ Tet spells (spells of cyanosis & hypoxemia) - Anoxic spells - Fatigue - Failure to Thrive (FTT): Decreased Caloric Intake At Risk For: - Emboli - Seizures - Loss of consciousness - Sudden death following an anoxic spell

Sickle Cell Anemia (SCA)

A genetic disorder that causes abnormal hemoglobin, resulting in some red blood cells assuming an abnormal sickle shape - Autosomal Recessive Disorder - Predominant in African-Americans Pathophysiology: - Partial or complete replacement of normal Hgb with abnormal Hgb S - Hgb in the RBC's takes on an elongated "sickle" shape - Can cause obstructions in vessels and heart - Hypoxia occurs and causes sickling Diagnosis: - Newborn screenings for SCD - Sickle-Turbidity test (Sickledex) - Hemoglobin electrophoresis ("fingerprinting") Clinical Manifestations: PAIN! - Painful swelling of hands & feet, painful joints - Severe pain resembling acute surgical condition - Stroke, visual disturbances - Symptoms resembling pneumonia, protracted episodes of pulmonary disease - Hematuria - Obstructive jaundice, hepatic coma - Priapism (persistent painful penile erection) - Chronic Anemia (HgB level of 6-9 g/dL) - Retinopathy - Avascular necrosis - Splenomegaly - Gallstones - Meaturia - Abdominal pain - Dactylitis (Hand-Foot Syndrome) - Chronic ulcers Therapeutic Management: - Rest to minimize energy expenditure & improve oxygen use - Hydration through oral or IV therapy - Electrolyte replacement because hypoxia results in Metabolic Acidosis - Analgesia for the severe pain from vasoocclusion - Blood replacement to treat progressing severe anemia - Abx therapy to treat any existing infection - Prophylactic antibiotics from 2 months - 5 years - Administration of Pneumococcal, Hib & Meningococcal vaccines - Monitor reticulocyte count to evaluate bone marrow function - Short-Term O2 (can suppress production of RBCs if prolonged!) - Splenectomy for Splenic Sequestration - Pain management - Rest to minimize energy loss - Folic Acid - Hydroxyurea (HbF/Maternal Hemoglobin Induction) Nursing Care Management: - Seek early intervention for problems such as fever 101.3F or higher - Give penicillin as ordered - Recognize S&S of stroke, splenic sequestration as well as respiratory problems that can lead to hypoxia - Treat the child normally - HYDRATION! (Fluids can come from soups, ice pops, ice cream, sherbert, gelatin & puddings) possible problem of Enuresis - Monitor child's growth; watch for FTT - Careful multisystem assessment - Assess Pain - Observe for presence of inflammation - Carefully monitor for signs of shock Psychosocial Needs: - Coping mechanisms - Support with genetic counseling - Caregiver role strain

von Willebrand Disease

A hereditary bleeding disorder, involving a deficiency of von Willebrand's factor (a plasma protein, and the carrier for factor VIII) - This factor is necessary for platelet adhesion - Transmitted as autosomal recessive OR dominant trait - Gene for disease is located on chromosome 12 Clinical Manifestations: - Easy bruising - Epistaxis - Gingival bleeding - Excessive bleeding with lacerations or surgeries - Menorrhagia Treatment: - Infusion of von Willebrand's protein concentrate - DDAVP infusion before surgery or to treat bleeding episode

Water Intoxication

A rare condition in which body water contents are too high in all body fluid compartments - "Water Overload" - Infants are especially vulnerable to this Causes: - IV water overloading - Too-rapid dialysis - Tap water enemas - Feeding of incorrectly mixed infant formula - Excessive water ingestion - Too-rapid reduction of glucose levels in DKA - Patients with CNS infections - Administration of inappropriate hypotonic solutions (ex. 0.45 sodium chloride) Clinical Manifestations: - Irritability - Somnolence - Headache - Vomiting - Diarrhea - Generalized seizures - Edematous

Cardiac Dysrhythmias

Abnormal heart rhythms - Listen to Apical for 1 minute Causes: - CHD - Electrolyte imbalances Diagnosis: - 12-Lead ECG - Cardiac Catheterization Classifications: - Bradydysrhythmias (Sinus bradycardia, Complete AV block) - Tachydysrhythmias (Sinus tachycardia, SVT)

Atrial Septal Defect (ASD)

Abnormal opening between the atria, allowing blood from the higher pressure left atrium to flow into the lower pressure right atrium - LEFT to RIGHT blood flow, Acyanotic - Defect with increased pulmonary blood flow Clinical Manifestations: - Patients may be Asymptomatic - Can lead to heart failure by the 3rd or 4th decade of life if undiagnosed - Murmur Surgical Closure: - Pericardial Patch/ Dacron patch - Transcatheter closure

Ventricular Septal Defect

Abnormal opening between the ventricles that allows blood to shunt back and forth - MOST COMMON DEFECT - LEFT to RIGHT blood flow, Acyanotic - Defect with increased pulmonary blood flow Clinical Manifestations: - Heart failure is common - Pulmonary Edema (pink frothy sputum) - Holosystolic Murmur (LSB) - Right-ventricular enlargement (Hypertrophy) Surgical Treatment: - Device closure

Hyperhemolytic Crisis

Accelerated rate of RBC destruction characterized by anemia, jaundice, and reticulocytosis - Type of sickle-cell crisis

Kawasaki Disease

Acute Systemic Vasculitis of an UNKNOWN cause - Mostly occur in children younger than 5 - Asian children have higher prevalence of this disease - Mucocutaneous lymph node syndrome - Autoimmune response Three Phases: Acute Phase: - Sudden high fever (104+ F), unresponsive to antipyretics and antibiotics - Child is very irritable - Labs show elevated WBC, LFTs and ESR & CRP Subacute Phase: - Lasts from the end of fever through the end of all KD clinical signs - Coronary artery aneurysms may be apparent, irritability persist - Labs show normochromic, normocytic anemia as well as thrombocytosis Convalescent Phase: - Clinical signs have resolved, but laboratory values have not returned to normal; ends when normal values have returned (6-8 weeks) Diagnosis: 1.) Changes in the extremities 2.) Bilateral conjunctival injection (inflammation) without exudation 3.) Changes in the oral membranes such as erythema, cracking of the lips, oropharyngeal reddening (Strawberry tongue) 4.) Rash: Maculopapular, diffuse erythroderma or erythema multiforme-like - Cervical lymphadenopathy (typically unilateral) Complications: Long-term complications include the development of Coronary Artery Aneurysms leading to: - Myocardial infarction (MI) - Myocardial ischemia Clinical Manifestations: - Red, dry cracked lips and inflamed tongue - Swollen lymph nodes - Strawberry Tongue - Widespread rash; diaper rash - Red eyes; Conjunctivitis - Skin peeling of the hands and feet - Fever (for more than 5 days) - CRASH & Burn Treatment: - High Dose IV Immunoglobulin (IVIG) w/Aspirin (Treatment outweighs the risk!) - If the child develops coronary abnormalities the Salicylate therapy/ Aspirin therapy is continued - Antithrombotic medications: Clopidogrel (Plavix), Enoxaparin (Lovenox), or Warfarin (Coumadin) Nursing Care Management: - Monitor I's & O's and daily weights are recorded - Fluids administered with care - Assessed frequently for signs of HF - Frequent cardiac assessments & vitals - Promote quiet environment that promotes adequate rest - Discharge teaching - Long-term follow up care

Blood Transfusion Therapy

Administration of a blood component Key Considerations: - Verify the identity of the recipient's and donor's blood group (2 pt identifiers) - Verify with a second HCP - Monitor vitals (baseline, initially; 15 min after initiation and completion) - Use blood within 30 minutes of arrival! - Infuse a full unit over 4 hours MAXIMUM! - Be alert to reactions; if a reaction occurs stop the transfusion IMMEDIATELY; have a secondary saline line ready Complications: - Hemolytic reactions (MOST SEVERE) - Febrile reactions - Allergic reactions - Circulatory overload (from too rapid infusion!) - Air emboli - Hypothermia - Electrolyte disturbances; HYPERKALEMIA (in massive transfusions or pt w/ renal problems) - Transmission of infection (delayed) - Alloimmunization Antibody formation (delayed)

Immune Thrombocytopenia

Also known as "Idiopathic thrombocytopenic purpura (ITP)"; an acquired hemorrhagic disorder characterized by: 1.) Thrombocytopenia 2.) Absence or minimal signs of bleeding (easy bruising, mucosal bleeding, petechiae) in most childhood cases 3.) Normal bone marrow with a normal or increased number of immature platelets (Megakaryocytes) and Eosinophils - ITP usually present 1-4 weeks after a viral illness and majority of cases are seen under 10 years old Types: - Newly diagnosed; less than 3 months - Persistent ITP that occurs from 3 months to 1 year - Chronic condition lasting longer than 12 months Clinical Manifestations: - Petechiae, ecchymoses (most often over bony prominences) - Epistaxis, bleeding gums - Internal hemorrhage evidenced by: Hematuria, hematemesis, melena, hemarthrosis, menorrhagia, hematomas over lower extremities Diagnosis: - Based on CM's - Platelet count less than 20,000/mm^3 - Tourniquet test, bleeding time & clot retraction are all abnormal Therapeutic/Nursing Management: - Primarily supportive; self-limiting disease - Prednisone, IV immunoglobulin & anti-D antibody - Premedication with acetaminophen 5-10 min before infusion of anti-D antibody is recommended - Restriction of contact sports while the child's platelet count is less than 50k

Biliary Atresia (BA)

Also known as extrahepatic biliary atresia; Abnormality in bile ducts: deformity/absent or narrowed Clinical Manifestations: - Jaundice persist beyond 2 weeks of age (especially if serum bilirubin is elevated!) - Dark urine - Stools acholic or gray - Hepatomegaly Diagnosis: - Hx & physical - CBC, bilirubin levels, LFTs - HIDA Scan Therapeutic/Nursing Management: - Nutritional support - Supplementation of fat soluble vitamins (A,D,E,K) - Surgical: Hepatic Portoenterostomy (Kasai Procedure)

Atrioventricular Canal Defect

Also referred to as AV septal defects or endocardial cushion defects; Incomplete fusion of the endocardial cushions - Allows blood from all 4 chambers of the heart to flow together - LEFT TO RIGHT shunting, Acyanotic - Defect with increased pulmonary blood flow Clinical Manifestations: - Moderate to severe HF - Loud Systolic Murmur - Cyanosis that increases with crying - High risk for developing pulmonary vascular obstructive disease Surgical Repair: - Pulmonary artery banding - Complete repair

Constipation

Alteration in the frequency, consistency or ease of passing stool - Children over 4 years old can be diagnosed if they have 3 or fewer stools a week - Constipation with fecal soiling is Encopresis - Usually idiopathic or functional Therapeutic Management: - Irrigation with hypertonic solution - Water-soluble enema - If due to HD, surgical treatment may be needed - Changing the diet to include more Fiber - Stool softener ners such as Malt Extract, Lactulose, Docusate Sodium, Polyethylene glycol (Miralax) - Mineral oil (1 year +)

Iron Deficiency Anemia

Anemia caused by an inadequate supply or loss of iron is the most prevalent and preventable nutritional disorder - Preterm infants are at risk because of their reduced fetal iron supply - Children with excessive intake of cow's milk (12-36 months) Preventable: - Iron-fortified cereals and formula for infants - Special needs of premature infants - Adolescents at risk Pathophysiology: - Primarily related to dietary intake - "Milk babies" (kids that only drink milk and not getting iron intake) Therapeutic/Nursing Management: - Increase the amount of iron the child receives (daily dose of iron should be given in 2-3 doses between meals, Ascorbic Acid/Vitamin C helps facilitate the absorption of iron) - IM Iron Injections (ONLY FOR SEVERE MALABSORPTION, can be dangerous & expensive) - Blood transfusions for severe anemia - Diet - Iron Supplementation (Can stain teeth, turn stool black, can cause upset stomach) - Patient education

Sickle Cell Crisis

Anything that increases body's needs for oxygen or alters transport of oxygen - Trauma - Infection, fever - Physical & emotional stress - Increased blood viscosity caused by Dehydration - Hypoxia Types: - Vaso-occlusive thrombotic - Splenic sequestration - Aplastic crisis - Hyperhemolytic Complications: - Acute chest syndrome - Infection - CVA (Stroke!)

Skin Lesions

Areas of pathologically altered tissue Causes: - Contact with injurious agents such as infective organisms, toxic chemicals and physical trauma - Hereditary factors -External factors such as allergens - Systemic diseases such as varicella, lupus and nutritional deficiency diseases - Children's age: birthmark malformations and dermatitis in early life, ringworm in school age children Diagnosis: - Objective characteristics of lesions and through careful inspection - Microscopic examination of skin, skin cultures, skin scraping or biopsy - Allergic skin testing - CBC Signs & Symptoms: - Itching/Pruritus - Pain & tenderness - Burning, prickling, stinging or crawling feelings - Absence of sensation (anesthesia) - Excessive sensitivity (hyperesthesia) - Diminished sensation (hypesthesia or hypoasthesia) - Abnormal sensations (paresthesia) - Erythema, ecchymoses (bruises) - Petechiae (Kawasaki's, Hemophilia, Thrombocytopenia) - Primary lesions, secondary lesions - Distribution pattern of the lesions, configuration & arrangement

Multisystem Inflammatory Syndrome in Children (MIS-C)

Associated with Coronavirus (COVID-19); Kawasaki disease but has more cardiovascular involvement - Different body parts become inflamed, including the heart, lungs, kidneys, bran, skin, eyes or G.I organs Pathophysiology: - Immune response to COVID-19 Clinical Manifestations: - Fever (>= 38 C) lasting 24 hours - Lab evidence of inflammation: Elevated CRP, ESR, fibrinogen, procalcitonin, ferritin, LDH or IL-6, elevated neutrophils, reduced lymphocytes and low albumin - Evidence of clinically severe illness requiring hospitalization with multisystem (2 organ involvement) - No alternative plausible diagnosis - Positive for COVID-19 (current or previously) within the 4 weeks prior to the onset of symptoms Therapeutic Management: - Similar to Kawasaki Disease - High Dose IV Immunoglobulin (IVIG) - Systemic glucocorticoids - Many children w this require mechanical ventilation & ECMO support

Hemodialysis

Blood is circulated outside the body by hydrostatic pressure exerted across a semipermeable membrane with simultaneous infusion of a replacement solution - Requires creation of a vascular access (Fistula) and special dialysis equipment - Best suited for children who can be brought to a dialysis facility three times a week for 4-6 hours - Achieves rapid correction of fluid and electrolyte abnormalities

Inflammatory Bowel Disease (IBD)

Can be 3 major forms of chronic intestinal inflammation: Crohn's Disease (CD), Ulcerative Colitis (UC) & Inflammatory Bowel Disease (IBDU) Causes: - Genetic factors - Children who migrate from Western countries - Small amount of intestinal bacterial species (overrepresentation & underrepresentation of some) Pathophysiology: Ulcerative Colitis: - Limited to the distal colon & rectum - Inflammation affects the mucosa & submucosa, varying degrees of ulceration, bleeding & edema - Toxic megacolon in SEVERE UC Crohn's Disease: (More Severe!) - Inflammation of ANY PART OF THE G.I TRACT from mouth to anus - Most often affects the terminal illeus - Involves all layers of the bowel wall (transmural) - May result in alterations, fibrosis, adhesions, stiffening of the bowel wall, stricture formation and fistulas Clinical Manifestations: Ulcerative Colitis: - Diarrhea with hematochezia - Bloody diarrhea, cramps, urgency with defecation, mild anemia, fever, anorexia, weight loss - Severe: Frequent blood stools, abdominal pain, significant anemia, fever & weight loss - Enlarged lymph nodes, arthritis and skin lesions Crohn's Disease: - Diarrhea, abdominal pain with cramps, fever & weight loss - Aphthous ulcers, peripheral arthritis, erythema nodosum, digital clubbing, renal stones and gallstones - Growth failure & delayed sexual maturation - Malabsorption & anorexia Diagnosis: - Hx & physical exam - CBC to evaluate anemia - ESR rate (usually elevated) - CRP to assess the systemic reaction to inflammatory process (usually elevated) - Fat soluble vitamins may be low in patients w/ CD - Stool analysis for blood, leukocytes and infectious organisms - Endoscopy & Colonoscopy w/ biopsy Therapeutic/Nursing Management: - 5-Aminosalicylates (5-ASAs) such as Mesalamine, Olsalazine & Balsalazide - Corticosteroids - Immunomodulators such as Azathioprine for children who are steroid resistant - Antibiotics: Metronidazole & Ciprofloxacin - Correction of nutritional deficits! (High-Protein, High-Calorie Diet) - NG infusions or TPN possible - Subtotal colectomy & ileostomy if nutritional therapies fail (UC)

Pet Bites

Children bitten by an animal; dog bites are most common Clinical Manifestations: - Varies from small puncture wounds to complete evulsion of the tissue that is associated with significant crush injury - S&S of infection Therapeutic Management: - Rinsing the wound with copious amounts of saline or LR solution under pressure via a large syringe, washing w/ mild soap - Clean pressure dressing is applied and the extremity is elevated if the wound is bleeding - Medical evaluation is advised because of the dangers of Tetanus & Rabies (usually from wild animals) Do NOT need Tetanus shot unless child had one within the last 5 years - Prophylactic antibiotics Nursing Care Management: - Prevention - Parents should monitor children's behavior with dogs and instruct them not to tease them or surprise the dog

Bacterial Skin Infections

Children with congenital or acquired immunodeficiency disorders such as AIDS, children receiving immunosuppressive therapy and those with malignancies like leukemia or lymphoma are at higher risks of developing these - Severity varies with skin integrity, immune and cellular defenses Causes: - Staphylococci or Streptococci bacteria - MRSA!!! Examples: - Impetigo contagiosa - Cellulitis Nursing Care Management: - Prevent the spread of infection & prevent complications - Hand hygiene!!! - Children w/ MRSA should be provided their own washcloths & towels separate from any family or other patients - Chlorine bath daily for 5-14 days - Tell parents NOT to squeeze follicular lesions; will spread infection - Mark localized area of infection on cellulitis on body part - Antibiotics & warm compresses for cellulitis

Tet Spells

Children with tetralogy of fallot exhibit bluish skin during episodes of crying or feeding Treatment: - Squatting position - Knee-chest position (helps blood flow back to the heart) - Oxygen - Morphine

Peptic Ulcer Disease (PUD)

Chronic condition that affects the stomach or duodenum Types: - Gastric Ulcer: involves the mucosa of the stomach - Duodenal Ulcer: involves the pylorus or duodenum - Primary Ulcers: idiopathic or associated with H.pylori - Secondary Ulcers: result from stress or severe underlying disease (severe burns, sepsis, increased ICP, trauma, MODS) Causes: - H.Pylori Infection - Ulcerogenic drugs, alcohol & smoking contribute to this - Stressful life events, dependency, passiveness & hostility have been implicated at contributing factors Clinical Manifestations: - Chronic abdominal pain especially when the stomach is empty such as during the night or early morning - Recurrent vomiting - Hematemesis (blood vomit) - Melena - Chronic anemia - Abdominal tenderness Diagnosis: - Hx, symptoms & physical exam - CBC to detect anemia - Stool analysis for occult blood - Liver function tests (LFTs) - ESR or CRP to evaluate IBD - Amylase & lipase to evaluate for pancreatitis - C13 breath test to test for H.pylori - Upper G.I series Therapeutic Management/Nurse Management: - Relieve discomfort, promote healing & prevent complications & prevent recurrence - Antacids - H2-receptor antagonist - PPI's : Omeprazole, Lansoprazole, Pantoprazole & Esomeprazole - Sucralfate: forms a barrier over ulcerated mucosa - Triple-drug therapy such as (1.) Bismuth (2.) Clarithromycin, (3.) Metronidazole - Surgical intervention for complications such as hemorrhage, perforation or gastric outlet obstruction - Acetaminophen for pain (NOT aspirin or NSAIDs) - Minimize stress!!!

Atopic Dermatitis (Eczema)

Chronic relapsing inflammatory skin disorder that results in itching & lesions 3 Forms: - Infantile: Usually begins at 2-6 months of age, generally undergoes spontaneous remission by 3 years of ago - Childhood: May follow the infantile form; occurs at 2-3 years of age; 90% of children have manifestations by age 5 - Preadolescent & Adolescent: Begins at about 12 years of age; may continue into the early adult years or indefinitely Diagnosis: - History & morphologic findings Clinical Manifestations: - Intense itching, unaffected skin dry & rough Infantile: - Erythema, vesicles, papules, weeping, oozing, crusting, scaling, often symmetric - Distribution: Generalized, especially in face, scalp, neck and extensor surfaces of extremities Childhood: - Symmetric involvement - Clusters of small erythematous or flesh colored papules or minimally scaling patches - Dry and may be hyperpigmented - Lichenification (thickened skin w/ attenuation of creases - Keratosis pilaris (follicular hyperkeratosis) common - Distribution: Flexural areas (antecubital & popliteal fossae, neck) wrists, ankles and feet Adolescent: - Same as childhood manifestations - Dry, thick lesions common - Confluent papules - Distribution: Face, sides of neck, hands, feet and antecubital & popliteal fossae Therapeutic Management: - Hydrate the skin!!! - Reduce flare-ups or inflammation - Relieve pruritus - Prevent & control secondary infection - Avoiding exposure to skin irritants or allergens, avoid overheating - Meds: Antihistamines, topical immunomodulators (2 years or older), topical steroids & sometimes mild sedatives as indicated - Tepid bath w/ Mild Soap (Dove or Neutrogena) no soap or an emulsifying oil followed immediately by the application of an emollient (within 3 minutes!) - Appropriate lotions to use: Aquaphor, Cetaphil & Eucerin - Non-sedative antihistamines such as Loratadine (Claritin) or Fexofenadine (Allegra) useful for daytime pruritus Nursing Care Management: - Examine the skin lesions - Ask the parents about child's history (if they are scratching, irritable & sleeping patterns) - Control the intense pruritus - One-piece outfits with long sleeves & long pants to decrease direct contact w/ the skin - Wash clothing in mild-detergent - Tell parents & child to keep good hygiene - Prevention of itching & infection

Seborrheic Dermatitis (Cradle Cap)

Chronic, recurrent inflammatory reaction of the skin - Occurs most commonly on the scalp (cradle cap) but may involve the eyelids (blepharitis) external ear canal (otitis externa), nasolabial folds & inguinal region - The cause is UNKNOWN, more common in early infancy when sebum production is increased - Lesions are thick, adherent, yellowlish, scaly, oily patches that may or may not be pruritic Nursing Care Mangement: - Scalp Hygiene - Teach parents how to shampoo the infant's hair & emphasize that the fontanel is similar to skin anywhere else on the body, it does not puncture or tear w/ mild pressure - Remove the scales or crust on the scalp when present - Antiseborrheic shampoo containing sulfur & salicylic acid

Nephrotic Syndrome

Clinical state that includes massive Proteinuria, Hypoalbuminemia, Hyperlipidemia & Edema - Usually affects children ages 1-8 years old Causes: 1.) Primary Disorder: Idiopathic nephrosis, childhood nephrosis or minimal-change nephrotic syndrome (MCNS) 2.) Secondary Disorder: Occurs as CM's after or in association with glomerular damage that has a known or presumed cause 3.) Congenital: Inherited as an autosomal recessive disorder Pathophysiology: - Normally is impermeable to large proteins but with NS it becomes permeable to proteins, especially albumin - Albumin is lost in the urine (hyperalbuminuria) - Serum albumin is decreased (hypoalbuminemia) - Fluid shifts from the plasma to the interstitial spaces (causing Hypovolemia & Ascites) Diagnosis: - Generalized edema (develops gradually or rapidly) - Proteinuria 2+ on dipstick testing - Hypoalbuminemia - Hypercholesterolemia (may or may not have Hypertension) - Low serum protein & sodium - Facial puffiness, Poor appetite - Oliguria (Normal output is 1-2 kg/hr for children) - Ascites - 24-Hour Urine Output! (will have SEVERE oliguria) Clinical Manifestations: - Weight gain, puffiness of face (facial edema) - Abdominal swelling (Ascites) - Pleural effusion - Labial or Scrotal swelling - Diarrhea, anorexia & poor intestinal absorption - Ankle or leg swelling - Irritability, easily fatigued, lethargic - BP normal or slightly increased - Susceptibility to infection - Oliguric, frothy urine Therapeutic/Nursing Management: - Reduce excretion of urinary protein (use Steroids) - Reduce fluid retention in tissues (use Diuretics) - Prevent infections (use Abx) - Minimize complications due to therapy - Corticosteroids are first line therapy for MCNS (Prednisone) - Children who do not respond well to steroid therapy and relapse multiple times will be given Immunosuppressive medications such as Cyclophosphamide (Cytoxan) - Prevent Infection!!! - Monitoring for fluid retention or excretion - Strict I's & O's - Applying & weighing diapers for output - Urine examination for albumin, daily weights and measurement of abdominal girth - Low to Moderate Protein Diet (with sodium restrictions when large amount of edema are present) - Frequent skin assessments for edema! - Routine & thorough perineal care for all hospitalized patients - Avoid unnecessary catheterization & early removal of indwelling catheters (prevent CAUTI) Family Support & Home Care: - Parents taught signs of relapse and call for changes in treatment at earliest indication - Test urine for albumin, administer meds and providing general care - Parents instructed to have the child avoid contact with infected playmates but they can still attend school - Social Isolation: Lack of energy, immunosuppression and protection for infection, change in appearance due to edema affects the self-image

Paraphimosis

Condition in which a retracted prepuce cannot be pulled forward to cover the glans - Inflamed & edematous

Hirschsprung Disease (HD)

Congenital anomaly that results in mechanical obstruction from inadequate motility of part of the intestine - Aganglionic Megacolon - 4x more common in males than in females Complications: - Enterocolitis Clinical Manifestations: Newborn: - Failure to pass meconium within 24-48 hours after birth - Refusal to feed - Bilious vomiting - Abdominal distention Infancy: - FTT - Constipation - Abdominal distention - Episodes of diarrhea & vomiting - Signs of enterocolitis (explosive & watery diarrhea, fever) Childhood: - Constipation - Ribbonlike, foul smelling stools - Abdominal distention - Visible peristalsis - Easily palpable fecal mass - Undernourished, anemic appearance Diagnosis: - In neonates on the basis of clinical signs of intestinal obstruction or failure to pass meconium - Hx & chronic pattern of constipation - Rectal examination (rectum is clear of feces, internal sphincter is tight, leakage of liquid stool) - Rectal Biopsy to CONFIRM Nursing Care Management: - If malnourished and cannot handle surgical intervention then symptomatic treatment (Enemas, Low-fiber, high-calorie, high-protein diet) - Temporary Ostomy & Pull-Through Procedure - Surgical intervention - Measure abdominal circumference

Epispadias

Congenital defect in which the urinary meatus is located on the Dorsal/Upper side of the penis

Foramen Ovale

Connects the two atria in the fetal heart - Helps the blood bypass the lungs, no oxygenation from fetal lungs until birth

Hypertrophic Pyloric Stenosis (HPS)

Constriction of the pyloric sphincter with obstruction of the gastric outlet - Develops in the first few weeks of life, causing nonbilious vomiting - If NOT diagnosed early: dehydration, metabolic alkalosis & FTT may occur Clinical Manifestations: - Nonbilious vomiting (early stages) usually 30-60 min after feeding and becomes Projectile Vomiting as obstruction progresses - Dehydration, Electrolyte imbalances - Weight loss - FTT - Olive-shaped mass in epigastrium - Hyperbilirubinemia Diagnosis: - Hx & physical exam - Olive-shaped mass easily palpated when stomach is empty - Ultrasound - Basal metabolic panel (ELECTROLYTES!!! Will be depleted!) - Decrease in chloride levels, Increase in pH & Bicarb - Elevated BUN from dehydration Therapeutic Management/Nursing Management: - Surgical relief of obstruction: Pyloromyotomy - Small feedings 4-6 hours post-op with water or electrolyte solutions - Assessment of eating behaviors & rise of CM's - Rehydrate & restore electrolyte imbalances (K+ replacement before surgery) - Assess vital signs frequently! - NG tube - IV fluids

Poison Ivy, Oak & Sumac

Contact with the dry or succulent portions of any of these 3 poisonous plants produces localized, streaked or spotty oozing and painful impetiginous lesions that are often highly urticarial Offending Substance: - Urushiol Therapeutic Management: - Calamine lotion, Soothing Burrow solution compress and/or Aveeno baths to relieve discomfort - Topical corticosteroid gel is effective for prevention or relief of inflammation; especially when applied before blisters form - Oral corticosteroids for severe reactions affecting the face, throat or genital region - Diphenhydramine Nursing Care Management: - The earlier the skin is cleansed the greater the chance of removing the urushiol before it attaches to the skin - Immediately flush the skin with COLD running water to neutralize the urushiol not yet bound to the skin (within 15 min) - All clothes that have come in contact with the plant must be removed with care and laundered in hot water & detergent Prevention: - Avoiding contact & removing the plant from the environment

Gastroesophageal Reflux (GER)

Defined as the transfer of gastric contents into the esophagus - Most frequently after meals and at night (differentiate from GERD!) - Peak incidence at 4 months of age, generally resolves spontaneously by 1 year of age Clinical Manifestations: Infants: - ALT or BRUE (Brief unexplained event) - Spitting up, regurgitation, recurrent vomiting (may be forceful) - Excessive crying, irritability, arching of the back, stiffening - Poor weight gain - Respiratory problems (cough, wheeze, stridor, gagging, choking w/ feeding) - Feeding refusal Children: - Heartburn - Abdominal pain - Chronic cough, hoarse voice - Dysphagia - Asthma - Recurrent vomiting Complications: - Esophagitis - Esophageal stricture - Laryngitis - Recurrent pneumonia - Anemia - Barrett esophagus Diagnosis: - Hx & physical exam - Infant Gastroesophageal Reflux Questionnaire - Upper G.I series - 24-Hour intraesophageal pH monitoring study - Endoscopy w/ biopsy - Scintigraphy Therapeutic Management/Nursing Management: - None needed for infant who is thriving w/ no respiratory complications - Avoidance of caffeine, citrus, tomatoes, alcohol, peppermint, spicy or fried foods - Feeding maneuvers (upright positioning for 30 min after feeding!, thickened feedings) - Constant NG feedings if severe - Prone positioning for infants (but increases risk for SIDS!) - H2-Receptor Antagonist: Ranitidine (Zantac) or Famotidine (Pepcid) - PPI's: Esomeprazole (Nexium), Lansoprazole (Prevacid), Omeprazole (Prilosec), Pantoprazole (Protonix) - Fundoplication surgical intervention for severe

Fungal Infections

Dermatophytoses (ringworm) are infections caused by a group of closely related filamentous fungi that invade primarily the stratum corneum, hair and nails - SUPERFICIAL INFECTIONS that live on the skin - Transmission from person to person or from infected animal to human Examples: - Tinea capitis (scalp) - Tinea corporis - Tinea pedis (feet) - Candidiasis (oral, diaper, vaginal) Nursing Care Management: - Contact Isolation Precautions! - Infected children should not exchange grooming items, headgear, scarves or other articles of apparel that have been close to the infected area - Given their own towels and directed to wear a protective cap at night to avoid transmitting infection to bedding - Ketoconazole & Selenium Sulfide shampoos may reduce colony counts (applied for 5-10 min 3x a week) - Griseofulvin drug therapy usually continues for weeks or months (take w/ high-fat foods for best absorption) - Antifungal medications (terbinafine, itraconazole, fluconazole)

Acquired Heart Disease (AHD)

Disease processes or abnormalities that occur after birth and can be seen in the normal heart or in the presence of congenital heart defects Factors Causing: - Infection - Autoimmune responses - Environmental factors - Familial tendencies

Esophageal Atresia (EA) & Tracheoesophageal Fistula (TEF)

EA: Failure of esophagus to develop as a continuous passage TEF: Failure of the trachea to separate into a distinct structure or abnormal connection between trachea & esophagus - May occur separately or in combination - Cause is unknown Clinical Manifestations: The 3 C's: Cough, Choking, Cyanosis EA: - Frothy saliva in mouth & nose - Drooling, choking & coughing - Respiratory distress - Child may cough or gag during feeding; Cyanosis & Apnea due to aspiration EA w/ TEF: - Stomach becomes distended with air - Thoracic & abdominal compressions (especially during crying) - Cyanosis or choking during feeding Diagnosis: - Chest radiographs - Presence of Polyhydramnios (accumulation of 2000 mL of amniotic fluid) is a clue Therapeutic/Nursing Management: - Managing patent airway! - Keep child NPO! - Prevention of aspiration pneumonia - Gastric or blind pouch decompression - Supportive therapy - Surgical repair - Pre & Post-op care

Scabies

Endemic infestations caused by the scabies mite Sarcoptes scabiei; Lesions are created as the impregnated female burrows into the stratum corneum of the epidermis to deposit her eggs and feces Clinical Manifestations: - Itching (most severe at night!) - Infants: Eczematous eruption, pruritus - Children: Minute grayish brown, threadlike (mite burrows), pruritus Distribution: - Generally in intertriginous areas (interdigital, axillary-cubital, popliteal & inguinal - Children older than 2: primarily in the hands & wrist - Children younger than 2: Primarily feet & ankles Nursing Care Management: - Scabicide (Permethrin) & supportive care - Prevent transmission - Keep child home and away from others - Wash clothing & bedding in VERY hot water

Hyperlipdemia

Excessive amounts of lipids in the blood - Identify kids at risk and treat early Risk Factors: - Positive family history of elevated cholesterol or early heart disease - Cigarette smoking - Obesity, sedentary lifestyle - Nutritional factors - Older age - Male gender - Hypertension - Type 1 or 2 diabetes Treatment: Lifestyle management - Restrict intake of cholesterol and fats - Increased intake of whole grains, fruits and vegetables - Exercise, stop smoking and avoid secondhand smoke - Statins: >10 years Pravachol (Pravastatin) > 8 - Antihyperlipidemic & Bile acid binding agents (Ezetimibe, Fibrates) Nursing Care Mangement: - Screening, education and support of the children & their families - Strict dietary changes need to be implemented

Cleft Lip & Cleft Palate

Facial malformations that occur during embryonic development - May appear separately or together - Closure of cleft palate typically done at 12-18 months - Effect on speech development Causes: - Multifactorial inheritance Closure of Lip Defect Precedes Correction of the Palate: - "Rule of Tens" (10 weeks, 10lbs, 10 Hgb) Therapeutic/Nursing Management: - Surgical correction - Risk of aspiration! - Pain management - Feeding (Cheek support when feeding, CANNOT BREASTFEED) - Pre & Post-Op care - Long-term care - Protect suture line - Special feeding equipment (wide based nipple for bottle feeding [Playtex])

Cryptochordism

Failure of one or both testes to descend through the inguinal canal into the scrotum - Anorchism: Absence of testes - May be related to an endocrine dysfunction - Common in preemies Clinical Manifestations: - Nonpalpable testes Diagnosis: - Distinguish between true undescended testis from more common retractile testes - Obviate the cremasteric reflex by placing the child in a squatting or cross-legged sitting position prior to checking the position of the testes Therapeutic/Nursing Management: - Hormone therapy with luteinizing hormone-releasing nasal spray - Human chorionic gonadotropin injection - Orchiopexy: Surgical intervention - Pain control - Child should avoid vigorous sports activities and use of toys that are straddled for 2-4 weeks post-op

Patent Ductus Arteriosus (PDA)

Failure of the fetal ductus arteriosus to close within the first week of life. - The continued patency of this vessel allows blood to flow from high pressure aorta to the lowe pressure pulmonary artery, which causes Left-to-Right shunt, Acyanotic - Defect with increased pulmonary blood flow Clinical Manifestations: - Machinery-Like Murmur - Wide Pulse Pressure, Bounding Pulses - Usually asymptomatic or show signs of HF Therapeutic Management: - Administration of Indomethacin/Ibuprofen(prostaglandin inhibitor) has proven successful in closing the PDA - Surgical repair via left thoracotomy - Transcatheter treatment

Vomiting

Forceful ejection of gastric contents through the mouth - Often accompanied by nausea and retching - Assess the color, amount and type of vomit - Fever & diarrhea accompanying this can mean Infection Complications - Dehydration can occur - Electrolyte disturbances - Malnutrition - Aspiration - Mallory-Weiss syndrome (small tears in the distal esophageal mucosa) Tx/Therapeutic Management: - Detection & treatment of the cause of vomiting - Antiemetic drugs like Ondansetron (Zofran) - Motion sickness medications such as Dimenhydrinate (Dramamine) Nursing Care Management: - Accurate assessment of the type of vomiting, appearance of the emesis and the child's behavior in association with the vomiting - Establish proper feeding methods - Position the child who is vomiting Upright to prevent observation & observe for S&S of dehydration - Monitor fluid & electrolyte imbalances - Brush teeth or rinse mouth after to dilute hydrochloric acid that comes in contact with the teeth

Diarrhea

Frequent passage of loose, watery stools Causes: Abnormal intestinal water and electrolyte transport - Rotavirus (Viral) - Norovirus (Viral) - E.coli - Salmonella groups - Shigella groups - Y. enterocolitis - C. jejuni - V. cholerae - C.difficile - C. perfringens - C. botulinum - Staphylococcus organisms Types: - Acute: Sudden increase in the frequency and a change of stools, often caused by an infection agent in G.I tract - Chronic: Increase in stool frequency and increased water content with duration for more than 14 days , often caused by malabsorption syndromes, IBD, immunodeficiency, food allergies & lactose intolerance Diagnosis: - History & physical assessment - Travel history & drinking water - Recent treatment with antibiotics - Dietary changes - Stool Cultures Nursing Care Management: - REHYDRATION!!! 1:1 Replacement! - Oral rehydration therapy (ORT) for infants and children - IV Fluids - Monitor for S&S of dehydration (especially the number of wet diapers or voidings) - Monitor I&O's - Regular Diet! Prevention: - Personal hygiene - Protection of contaminated water supply - Careful food preparation - Traveling precautions - Rotavirus Vaccine!!!

Celiac Disease

Gluten-Sensitive Enteropathy; Autoimmune disorder triggered by the ingestion of Gluten in genetically susceptible individuals - Usually appears 6-24 months Clinical Manifestations: Symptoms arise when solid foods such as beans & pasta are introduced into the diet at ages of 1-5 years old - Steatorrhea - Exceedingly foul-smelling stools - Malnutrition - Muscle wasting - Anemia, anorexia - Abdominal distention - Irritability, uncooperativeness, apathy Celiac Crisis: - Severe episodes of profuse watery diarrhea & vomiting - May be precipitated by infections, prolonged fluid & electrolyte depletion & emotional disturbances Complications: - Villous Atrophy Diagnosis: - Serologic blood test for tissue Transglutaminase & Antiendomysial antibodies in children 18 months of age or older (positive markers followed up by upper GI endoscopy w/ biopsy) Therapeutic/Nursing Management: - Gluten-Free Diet! - Creating a dietary regimen - Advise patient to read all food labels carefully - Encourage parents & child to find new gluten free recipes

Systemic Hypertension

High Blood Pressure - Essential or Secondary - Pediatric hypertension is generally secondary to a structural abnormality or an underlying pathologic condition Recommendations: - BP routinely checked 3 years + - Auscultation preferred method Diagnosis: - Based on percentile for sex, age and height on three or more occasions Classifications: - Normal: <90th Percentile - Prehypertension: 90th to <95th percentile or if BO exceeds 120/80 mmHg even if below the 90th percentile up to <95th percentile - Stage 1: 95th percentile to 99th percentile + 5 mmHg - Stage 2: > 99th percentile + 5 mmHg Treatment: - Beta-blockers (-lol) - Calcium channel blockers

Heart Failure

In children, occurs as a result of structural abnormalities - Right or Left-Sided - Heart muscle may become damaged if left untreated Clinical Manifestations: - Impaired myocardial contractility: (Tachycardia, diaphoresis, decreased urinary output, fatigue, weakness, restlessness, anorexia, pale, cool extremities, decreased BP, gallop rhythm, cardiomegaly) - Pulmonary congestion (LEFT): (Tachypnea, dyspnea, retractions, flaring nares, exercise intolerance, orthopnea, cough & hoarseness, cyanosis, wheezing, grunting) - Systemic venous congestion (RIGHT): (Weight gain, hepatomegaly, peripheral edema, especially periorbital, ascites, neck vein distention) Diagnosis: - Made on the basis of clinical symptoms (tachycardia & tachypnea at rest, dyspnea, retractions, activity intolerance, feeding intolerance, weight gain caused by fluid retention and hepatomegaly) - X-Ray, ECG, Echo - Daily Weights Treatment: IMPROVE CARDIAC FUNCTION - Cardiac Glycosides (Digoxin) : Monitor potassium levels, do NOT give if pulse rate is LOW - ACE-Inhibitors (-pril) - Beta-Blockers (-lol) Remove Accumulated Fluid & Sodium - Furosemide, Spironolactone,Thiazides (Chlorothiazide/Diuril) - Decreased cardiac demands: Activity restrictions, providing a neutral thermal environment to prevent cold stress in infants, treating any existing infections and reducing the effort of breathing - Improved tissue oxygenation (Give O2) Nursing Care Management: - Reduce cardiac demands that strain the failing heart muscle - Digoxin (check apical pulse & potassium levels before administration!) - Minimize fluid overload - Reduce afterload - Plan feeding to accomodate a child's wake-sleep cycle - Monitor temperature closely for hypothermia - Change position q2h to check for skin breakdown! - Assess oxygenation status and respiratory system! (count respirations for 1 full minute!) - Make sure child is well rested before feeding (3hr feeding cycle works best for most infants) - Support the child & family

Meconium Ileus

In cystic fibrosis, meconium plug obstructs intestine preventing stool passage at birth Tx/Management: - Irrigations with hypertonic solution or contrast medium - Early surgical intervention may be needed to evacuate the small intestine

Congenital Heart Disease (CHD)

Includes primarily anatomic abnormalities present at birth that result in abnormal cardiac function - Pressure always goes from HIGHER to LOWER Symptoms: Symptoms may appear 4-12 weeks after birth - FTT: Increase Metabolism (poor weight gain, activity intolerance) - Developmental delays - Prenatal history - Positive family history of cardiac disease Two Categories: - Heart Failure (HF) - Hypoxemia Types: - Acyanotic defects - Cyanotic defects Blood Flow Patterns: - Increased pulmonary blood flow (Acyanotic) - Decreased pulmonary blood flow (Cyanotic) - Obstruction to blood flow (Acyanotic) - Mixed blood flow (Cyanotic) Physical Exam: - Inspection (ex. cyanosis, poor perfusion, cold skin, delayed capillary refill, lifts/heaves, chest deformities, abnormal pulsations) - Palpation & Percussion (ex. high graded murmurs, bounding pulses in upper extremities) - Auscultation (listening for extra heart sounds, murmurs) Diagnosis: - ECG - Echo - Cardiac Catheterization

Maintaining Water Balance

Increased Water Requirements: - Fever - Vomiting, diarrhea - High-output kidney failure - Diabetes insipidus (DI) - Diabetic ketoacidosis (DKA) - Burns - Shock - Tachypnea - Radiant warmer (preterm infants) - Phototherapy (infants) - Postoperative bowel surgery (ex. gastroschisis) Decreased Requirements: - Heart failure - Syndrome of inappropriate antidiuretic hormone (SIADH) - After surgery - Oliguric renal failure - Increased ICP

Urinary Tract Infection (UTI)

Infection of the urinary tract - One of the most common childhood conditions - Most important host factor is urinary stasis - Uncircumcised males are at a higher risk Classifications: - Bacteriuria: Presence of bacteria in urine (Asymptomatic: No clinical symptoms, Symptomatic: Clinical signs present like dysuria, discomfort, hematuria and fever) - Pyuria: Presence of WBC in urine - Recurrent: Repeat episodes of UTI w/ same strain of bacteria - Persistent: Persistence of bacteriuria despite Abx treatment - Frequent: More than 3 UTI's within 6-months; does not have to be same strain - Febrile: UTI accompanied by a fever and other S&S (usually implies pyelonephritis) - Cystitis: Inflammation of the bladder - Urethritis: Inflammation of the urethra - Pyelonephritis: Inflammation of the upper urinary tract and kidneys; kidney infection (includes fever) - Urosepsis: Febrile UTI coexisting with systemic signs of bacterial illness; blood culture reveals presence of urinary pathogens Causes: - E.coli is most common pathogen! - Prevalence higher in females (shorter urethra) then males Clinical Manifestations: Neonate (Birth to 1 Month) - Poor feeding - Vomiting - Failure to gain weight - Rapid respirations (acidosis) - Respiratory distress - Frequent urination - Screaming on urination - Jaundice - Fever Infancy (1-24 Months) - Poor feeding, vomiting, excessive thirst, frequent urination, Straining or screaming on urination - Foul-smelling urine - Palor, fever, persistent diaper rash - Dehydration , enlarged kidney or bladder Childhood (2-14 years old): - Poor appetite, vomiting, growth failure, excessive thirst, enuresis, incontinence, frequent urination, painful urination - Swelling of face, pallor, fatigue - Blood in urine (hematuria) - Abdominal or back pain - Hypertension, edema, tetany Diagnosis: - Urine Culture, Urinalysis - Clean catch specimen is preferred - BUN & Creatinine - Suprapubic Aspiration (for children younger than 2) performed by bladder catheterization - Urine dipsticks - Ultrasound, Voiding Cystourethrogram (VCUG/VCG), DMSA scan Therapeutic/Nursing Management: - Eliminate the infection - Identify contributing factors to reduce the risk of recurrence - Prevent systemic spread of infection - Preserve renal function - Antibiotic Therapy! (Penicillins, Sulfonamide, Cephalosporins, Nitrofurantoin) - Parent's clean child's genital area properly, (wipe front to back after voiding & defecating) - Educate parents and child to void as soon as they feel the urge and should NOT hold in for long periods of time Teaching: - Maintaining adequate fluid intake - Emptying bladder with urination - Urinating before & after sexual intercourse - Acidic beverages help - Cotton undergarments

Pediculosis Capitis (Head Lice)

Infestation of the scalp by Pediculus humanus capitis a common parasite in school-age children Clinical Manifestations: - Pruritus (caused by crawling insects & insect saliva on skin) - Nits observable on hair shaft Distribution: - Occipital area - Behind ears - Nape of the neck - Eyebrows & eyelashes Diagnosis: - Observation of the white eggs (nits) firmy attached to the hair shafts Therapeutic Management: - Permethrin Cream Rinse (Nix) which kills adult lice & nits; second treatment at 7-10 days to ensure a cure - Malathion; not recommended in children younger than 2 Nursing Care Management: - Surveillance & education - Preventing spread & recurrence

Endocarditis

Inflammation of the Endocardium (lining of the heart) - Bacterial (BE) & Subacute Bacterial (SBE) are now referred to as Infective Endocarditis (IE) - OFten a sequel of bacteremia in children with CHD or AHD Causative Agent: - Streptococcus viridans - Staphylococcus aureus Clinical Manifestations: - Unexplained fever (usually low grade or intermittent) - Anorexia, malaise, weight loss - Osler nodes (red, painful intradermal nodes found on the pads of the phalanges) - Janeway lesions ( painless hemorrhagic areas on the palms and soles) - Petechiae on oral membranes - Splinter hemorrhages under the nails - May be present: HF, cardiac dysrhythmia, new murmur or change in previous one Diagnosis: - Based on clinical manifestations; Dukes Criteria - Growth & identification of causative agent in the blood - Anemia, elevated ESR, leukocytosis, microscopic hematuria may suggest IE Treatment: - High doses of antibiotics for 2-8 weeks! - Blood cultures are taken periodically for antibiotic therapy - Antibiotics (Penicillin) - Prophylactic antibiotics before procedure 1 hour before (Amoxicillin, ampicillin & clindamycin) - Prophylactic antibiotics for high risk patients Nursing Care Management: - Prophylactic therapy for dental work! - High levels of oral health in children with heart disease! - Teach parents & child signs and symptoms of of infection & to report any unexplained symptoms - IV antibiotic administration at home

Acute Pyelonephritis

Inflammation of the Upper Urinary Tract & Kidneys - Recurring episodes lead to a scarred, poorly functioning kidney & chronic pyelonephritis Causes: - E.coli, P.Klebsiella & Enterobacter bacteria Diagnosis: - Urine Culture, Urinalysis - Clean catch specimen is preferred - BUN & Creatinine - Urine dipsticks - Ultrasound, Voiding Cystourethrogram (VCUG/VCG), DMSA scan Clinical Manifestations: - N/V, anorexia, chills, nocturia, frequency & urgency - Suprapubic or low back pain & dysuria - Fever, hematuria and foul-smelling urine - Costovertebral Tenderness (CVA) Therapeutic/Nursing Management: - Hospitalization - Parenteral Abx - Observe for signs & symptoms of UTI - Monitor I's & O's - Collet urine specimen - Monitor labs - Administer meds - Education on prevention

Acute Appendicitis

Inflammation of the vermiform appendix - Most common cause of emergency abdominal surgery in childhood - Peak incidence between 12-18 years old (boys more affected than girls) - Can perforate within 48 hours of initial complaint of pain! Perforation Complications: - Major abscess - Phlegmon - Enterocutaneous fistula - Peritonitis - Partial bowel obstruction Cause: - Obstruction in the lumen of the appendix usually by hardened fecal matter - Swollen lymphoid tissue frequently occuring after a viral infection can also obstruct this - Enterobius vermicularis (pinworms) but this is RARE Clinical Manifestations: - RLQ abdominal pain (Pain @ McBurney's Point!) - Fever - Rebound tenderness (tend not to do w/ children because it's very painful) - Rigid abdomen - Decreased or absent bowel sounds - Vomiting (typically follows onset of pain) - Constipation or diarrhea - Anorexia - Tachycardia - Rapid, shallow breathing - Pallor, lethargy, irritability - Stooped posture - IF RUPTURED: Goes from Pain to NO PAIN!!! (Emergency!) Diagnosis: - Hx & physical examination - Pain (cardinal feature) is initially generalized in the periumbilical area (RLQ) most intense pain at McBurney's point - Rebound tenderness (can be extremely painful!!!) - CBC, Urinalysis to rule out UTI - hCg to rule out pregnancy in females - Ultrasound, CT scan Therapeutic Mangement/Nursing Management: - Hx & physical exam - Surgical removal of the appendix (Appendectomy) - Frequent pain assessments! (VERY LIGHT PALPATION OF ABDOMEN) - Ask the child with mild pain to lift the hells and drop them off the floor 2-3x or hop on one foot or to "puff out" or "pull in" the abdomen to check for tenderness without painful probing - IVF & Antibiotics (pre & post-op) - If drain is placed post-op then frequent skin assessments & dressing changes - PAIN MANAGEMENT ; analgesics are given regularly - Early ambulation after surgery! - Use of incentive spirometry! - No contact sports or heavy lifting! - Frequent assessment of bowel sounds & pain!

Rheumatic Fever (RF)

Inflammatory disease occurs after Group A B-Hemolytic Streptococcal (GAS) Pharyngitis (Antistreptolysin O titer (ASO)) - Can LEAD TO "Rheumatic Heart Disease (RHD) -Affects joints, skin, brain, serous surfaces and heart - Aschoff bodies/nodules on heart valves - Mitral valve is most often affected Causes: - Upper respiratory tract infection with GAS Diagnosis: - Jones Criteria Clinical Manifestations: CRP, ESR Major: Carditis - New murmur of valve regurgitation (mitral most common) - Tachycardia out of proportion to fever - Pericardial friction rub - Chest pain - Muffled heart sounds - Cardiomegaly on chest x-ray - Prolonged PR interval on ECG Polyarthritis - Sydenham Chorea - Swollone, hot, red, very painful joints - Migatory - large joints - Sudden aimless, irregular movements of extremities, exacerbated by stress - Emotional lability - Facial grimaces & speech disturbances - Erythema marginatum Minor: - Arthralgias - Fever (greater than 38.5 C) - ESR above 60 mm and/or CRP greater than 3 mg/dL - Prolonged PR interval Therapeutic Management: - Eradication of strep throat - Manage fever & inflammation - PRevention of permanent cardiac damage - Palliation of other symptoms - Preventions of recurrence of RF Treatment: - Treatment of strep tonsillitis and pharyngitis - Penicillin - Aspirin (Tx outways the risk! Only time to use in children!) - HF therapies such as oxygen, diuretics, fluid & salt restrictions, digoxin or ACE's - Children w/ chorea with motor impairment must be protected from injury & eliminate physical & emotional stress - Prophylactic treatment and prevention of RHD; IM injections of benzathine penicillin G every 28 days Nursing Care Management: - Encourages compliance with drug regimens - Facilitates recovery from the illness - Provides emotional support - Educating the parents to follow up with antibiotic prophylaxis (NEED COMPLIANCE & TAKING THE FULL DOSE)

Acne

Inflammatory disease of the skin involving the sebaceous glands and hair follicles - Predominantly in adolescents Patho: - Excessive sebum production - Alterations in follicular growth - An accompanying immune response to inflammation - Propionibacterium acnes (inflammatory reaction) TherapeuticManagement: - Clean the skin regularly (twice daily!) - Meds: Retin-A (topical), OCP, Accutane Nursing Care Management: - Nurse should assess the individual's level of distress, current management and perceived success of any regime before initiating a referral - Educate adolescent on factors that aggravate the skin such as too vigorous scrubbing

Contact Dermatitis

Inflammatory reaction of the skin to chemical substances, natural or synthetic that evoke a hypersensitivity response or direct irritation - Most commonly in the face & neck, back of the hands, forearms, male genitalia and lower legs Causes: - Primary Irritant (often that irritates any skin) - Sensitizing Agent ( produces an irritation on those individuals who have encountered the irritant or something chemically related to it, have undergone an immunologic change and have become sensitized) Common Sources: - Plant: Poison ivy, oak - Animal: Wool, features, furs - Metal Irritants: Nickel - Fabrics, dyes, perfumes, soaps Therapeutic/Nursing Management: - Prevent further exposure - Symptomatic care - Calamine lotion, Hydrocortisone cream for pruritus - Identify offending agent - Cleanse skin - Prevention

Thalassemia

Inherited blood disorders of Hemoglobin synthesis; Deficiencies in the rate of production of globin chains in the Hgb - Advantageous trait to protect against malaria - Affects mainly descendants of people living near the Mediterranean sea - Autosomal recessive Classifications: - Major (Cooley anemia/most common) - Minor (mild microcytic anemia) Clinical Manifestations: - Pallor, unexplained fever, poor feeding, enlarged spleen or liver (Before diagnosis) - Signs of chronic hypoxia (headache, precordial & bone pain, decreased exercise intolerance, anorexia) - Small stature, delayed sexual maturation, bronzed, freckled complexion - Enlarged head, prominent frontal & parietal bossing, prominent malar eminences, flat or depressed bridge of the nose, enlarged maxilla, generalizes osteoporosis Diagnosis: - Hematologic studies reveal characteristic changes in RBC's - Low Hgb & Hct levels - Hgb Electrophoresis Therapeutic Management: - Splenectomy Blood Transfusions (Major) crucial to maintaining Hgb level of 9.5 g/dL, every 2-5 weeks - Improved physical & psychological well being - Decreased cardiomegaly & hepatosplenomegaly - Fewer bone changes - Normal or near-normal growth & development until puberty - Fewer infections - Complications of Blood Transfusions: Hemosiderosis (iron overload) use iron chelating agents to minimize the development such as Deferoxamine, Deferasirox & Deferiprone Nursing Care Management: - Promote compliance with transfusion & chelation therapy - Assist the child in coping with anxiety-provoking treatments & the effects of the illness - Foster the child's family & adjustment to chronic illness - Observe for complications of multiple blood transfusions

Cardiac Catheterization

Invasive diagnostic procedure in which a radiopaque catheter is introduced through a large-bore needle into a peripheral vessel (usually the femoral artery or vein in child) and then guided into the heart with the air of fluoroscopy - Contrast material is injected and images are taken of circulation (Angiography) Types: - Diagnostic - Interventional - Electrophysiology studies Preprocedural Care: - Assess and mark pulses - Baseline O2 sat - NPO Postprocedural Care: - Check the pulse distal to the site - Monitor temp & color of extremities - Take vital signs every 15 min - Monitor BP - Monitor the dressing for bleeding or hematoma - Monitor intake and output - Keep extremities straight 4-6 (Venous) and 6-8 (Arterial)

Compartment Syndrome

Involves the compression of nerves and blood vessels due to swelling within the enclosed space created by the fascia that separates groups of muscles 6 P's of Neurovascular Assessment: - Pain - Poikilothermia (affected area should be same temp as rest of the body) - Paresthesia - Paralysis - Pulselessness - Pallor

Acute Kidney Injury (AKI)

Kidneys are unable to regulate the volume & composition of urine appropriately in response to food and fluid intake in the needs of an organism - Principle features include: Oliguria, Azotemia, Metabolic Acidosis & Diverse Electrolyte Disturbances Causes: - Acute Glomerulonephritis (AGN) - Hemolytic Uremic Syndrome (HUS) - Poor renal perfusion, urinary tract obstruction, acute renal injury, cardiac surgery or the final expression of chronic, irreversible renal disease - Severe Dehydration Clinical Manifestations: - Oliguria - N/V - Drowsiness - Edema - Hypertension Therapeutic/Nursing Management: - Treatment of the underlying cause - Management of the complications of renal failure - Provision of supportive therapy within the constraints imposed by the renal failure - Volume Restoration/ Rehydration!!! - Insertion of Foley catheter to rule out urine retention & to collect urine sample - Monitor Strict I's & O's - Frequent assessment of vital signs (observe for complications) - Dialysis (Hemo or Peritoneal) - Fluid Restrictions! - Optimal thermal environment - Supportive/Empathetic therapy - Neurochecks! Complications: HYPERKALEMIA (K+ > 5.3 mEq/L!!!) - Administer Polystyrene Sulfonate (Kayexalate), Dialysis, Albuterol (better in children than insulin) Hypertension - Frequent BP assessments q4-6 - Antihypertensive drugs - Limit fluids & salt Anemia - Hemoglobin < 6 g/dL - Transfusions of fresh, packed RBC's given SLOWLY to reduce the risk of increasing blood volume, hypertension & hyperkalemia Seizures - Usually related to Hypertension, Hyponatremia & Hypocalcemia - Correct underlying issues Cardiac Failure - Due to pulmonary edema & hypervolemia - Reduction of fluid volume with water & sodium restrictions - Diuretics

Acute Splenic Sequestration

Large amounts of blood pool in the spleen causing signs of hypovolemic shock S&S: - Irritability - Unusual sleepiness - Looks pale - Weakness - Fast heart beat - Splenomegaly - Pain on the left side of abdomen Tx: - SPLENECTOMY!

Coarctation of the Aorta

Localized narrowing near the insertion of the ductus arteriosus, which results in increased pressure proximal at the defect (head & upper extremities) and decreased pressure distal to the obstruction (body & lower extremities) - LEFT TO RIGHT, Acyanotic - Obstructive Defect Clinical Manifestations: - High BP & Bounding pulses in the arms - Weak or absent femoral pulses - Cool lower extremities with lower BP - Can deteriorate rapidly with severe acidosis & hypotension - Dizziness, headaches, syncope, epistaxis (from Hypertension) At Risk For: - Hypertension - Ruptured aorta - Aortic aneurysm - Stroke Treatment: - Surgical repair - Transcatheter treatment

Short Bowel Syndrome (SBS)

Malabsorptive disorder resulting from decreased mucosal surface area, usually as a result of a small bowel resection - Complication of surgery from Malrotation & Volvulus! Two Important Findings: 1.) Decreased intestinal surface area for absorption of fluid, electrolytes and nutrients 2.) A need for parenteral nutrition (PN) Therapeutic/Nursing Management: - Preserving as much length of bowel as possible during surgery - Maintaining optimum nutritional status, growth & development - Stimulating intestinal adaption with enteral feeding - Minimizing complications related to disease process & therapy - Nutritional Support with PN & Enteral Feeding - Closely monitor serum vitamin & mineral levels - Routine Ostomy care

Malrotation & Volvulus

Malrotation: Abnormal rotation around the superior mesenteric artery during embryonic development Volvulus: Twisting of the intestine around itself, compromising the blood supply to the intestines Diagnosis: - Hx & physical - Plain abdominal radiograph & lateral decubitus view obtained - Upper G.I series Therapeutic/Nursing Management: - Surgery to remove the affected area - IV fluids, NG decompression - Systemic Antibiotics Complication of Surgery: - Short Bowel Syndrome

Lyme Disease

Most common tickborne disorder, caused by the spirochete Borrelia burgdorferi which enters the skin and bloodstream through saliva and feces of ticks, especially the deer tick - Most commonly occur in children 5-15 years old Clinical Manifestations: First Stage: (3-30 days) - Tick bite ; Erythema migrans at the site of the bite - Bite enlarges radially up to 30 cm (12 inches) over a period of days to weeks - Large circumferential ring with raised edematous doughnut-like bred resulting in a bull's eye appearance (common in thigh, groin and axilla) - Fever, myalgia, headache or malaise Second Stage: (3-10 weeks after inoculation) - Smaller, secondary annular lesions without the indurated center (occur anywhere except on palms & soles of feet) - Fever, headache, malaise, fatigue, anorexia, stiff neck, generalized lymphadenopathy - Splenomegaly - Conjunctivitis, sore throat, abdominal pain and cough Third Stage: (2-12 months after inoculation) - Lyme Arthritis is most common manifestation with pain, swelling and effusion Rare Neuro: Meningitis, encephalitis and polyneuritis - Cardiac: acute atrioventricular conduction abnormalities, pericarditis or mild left ventricular dysfunction Diagnosis: - Observation of the lesion & CM's - Screening test enzyme immunoassay or immunofluorescence immunoassay - Western immunoblot testing Therapeutic Management: - Oral Doxycycline, Amoxicillin (children under 8) - Treatment usually last 14-21 days - IV antibiotics such as Ceftriaxone or Penicillin G (for Neuro, Cardiac or Arthritic manifestations) Nursing Care Management: - Educating parents on preventing exposure to ticks, children should avoid tick-infested areas or wear light-colored clothing so that ticks can be spotted easily, tuck pant legs into socks and wear long-sleeved shirt tucked into pants when in wooden areas - Education on tick removal (uses tweezers, grasp tick near the mouth parts and close to skin as possible) - Use of insect repellents containing Diethyltoluamide (DEET) and permethrin

Pulmonic Stenosis

Narrowing at the entrance to the Pulmonary Artery - Resistance to blood flow causes right ventricular hypertrophy & decreased pulmonary blood flow - LEFT TO RIGHT, Acyanotic - Obstructive Defect Clinical Manifestations: - Patients may be Asymptomatic; some have mild cyanosis or HF - Right Ventricular Hypertrophy - Cyanosis - Murmur At Risk For: - Infective endocarditis

Aortic Stenosis

Narrowing of the Aortic Valve - Causing resistance to blood flow in the Left Ventricle, decreased cardiac output, left ventricular hypertrophy & pulmonary vascular congestion - LEFT TO RIGHT, Acyanotic - Obstructive Defect Clinical Manifestations: - Left Ventricular Hypertrophy - Pulmonary Edema - Decreased C.O Newborns: - Decreased cardiac output w/ Faint Pulses - Hypotension - Tachycardia - Poor feeding Children: - Exercise intolerance - Chest pain - Dizziness when standing for a long period - Murmur At Risk For: - Infective endocarditis - Coronary insufficiency - Ventricular dysfunction

Phimosis

Narrowing or stenosis of the preputial opening of the foreskin that prevents retraction of the foreskin over the glans penis - Usually resolves on its own as child grows and the distal prepuce dilates Nursing Care Mangement: - Proper hygiene of the phimotic foreskin - Foreskin should not be forcibly retracted Treatment: - Topical corticosteroids - Circumcision

Epistaxis

Nosebleeds; especially from the septum - Recurrent or severe episodes may indicate an underlying disease (vascular abnormalities, leukemia, thrombocytopenia, clotting factor deficiencies like hemophilia or von Willebrand's) Causes: - Direct trauma including blows to the nose, foreign bodies and nose picking - Mucosal inflammation associated w/ allergic rhinitis and URI Nursing Care Management: - Remain calm, keep child calm - Have child sit up and lean forward - Put pressure to nose - Bleeding usually stops within 10 minutes after nasal pressure - Further evaluation needed if bleeding exceeds 10 minutes

Dehydration

Occurs whenever the total output of fluid exceeds the total intake, regardless of the underlying cause - Common body fluid disturbance in nursing care of infants & young children - "Volume Depletion" Causes: - Diarrhea/Vomiting - Decreased oral intake - DKA - Extensive burns Types: - Isotonic: Electrolytes & water deficits are BALANCED - Hypotonic: Electrolyte deficit exceeds the water deficit - Hypertonic: Water loss exceeds the electrolyte deficit (MOST DANGEROUS) Assessment of Hydration Status: - Urine & stools - Vomitus - Dry, chapped lips (early sign!) - Vital signs - Dry mucous membranes! (moderate sign!) - Body weight & strict I&O's (diaper weights) - Fontanels (sunken or bulging) - Poor Skin Turgor! (Late sign!!!) - Tears - Sensory alterations - Tissue perfusion - Labs Diagnosis: - Depends on the degree, based on physical assessment - Initial plasma sodium concentrations - Serum bicarbonate concentrations - Any associated electrolyte (especially serum K+) and acid-base imbalances (as indicated) Nursing Care Management: - Correcting fluid imbalance and treating underlying cause - Oral Rehydration (Oral Rehydration Solution 50 mL/kg) - Administer Ondansetron (Zofran) for N/V - IVF (usually 0.9% NaCl or LR)

Tumor Lysis Syndrome (TLS)

Oncologic emergency that is a result of the malignancy/ aggressive treatment; Rapid release of intracellular contents during the lysis of malignant cells - Usually occurs in patients with Acute Lymphoblastic Leukemia or Burkitt Lymphoma Metabolic Abnormalities: - Hyperuricemia (can lead to renal failure!) - Hypocalcemia - Hyperphosphatemia - Hyperkalemia Risk Factors: - High WBC count at diagnosis - Large tumor burden - Cancer cell sensitivity to chemotherapy - High proliferative rate S&S: - Flank pain - Lethargy - N/V - Muscle cramps - Pruritus - Tetany & seizures (from Hypocalcemia) Therapeutic/Nursing Care Management: - Early identification of patients at risk - Prophylactic measures & early interventions - Serum chemistries & urine pH monitored frequently - Strict I's & O's - Aggressive administration of IV fluids - Allopurinol to reduce uric acid formation, Urate Oxidase or Rasburicase - Exchange transfusion

Aplastic Anemia

Rare & life-threatening disorder; bone marrow failure condition in which the formed elements of the blood are simultaneously depressed Causes: - Congenital (Fanconi syndrome) - Acquired (usually idiopathic) Diagnosis: - Onset of CM"s such as Anemia, Leukopenia, Thrombocytopenia - Bone marrow examination (conversion of red bone marrow to yellow, fatty bone marrow) Camitta Criteria: Less than 25% bone marrow cellularity w/ at least two of the following findings - Absolute granulocyte count less than 500/mm^3 - Platelet count less than 20,000/ mm^3 - Absolute reticulocyte count less than 40,000/mm^3 Therapeutic/Nursing Management: - Immunosuppressive therapy (IST) to remove the presumed immunologic functions that prolong aplasia - Replacement of the bone marrow through transplantation - Antilymphocyte globulin (ALG) or Antithymocyte globulin (ATG) are drugs of choice for IST - Transfusions more successful when performed before multiple transfusions have sensitized the child to leukocyte & Human Leukocyte Antigens (HLA) - Prepare child & family for procedures - Monitor IV site to prevent extravasation - Use of chemotherapy agents in the treatment of relapsed patients, know the S&S such as (N/V, alopecia, mucositis)

Exstrophy-Epispadias Complex

Rare, complex disorder characterized by the dorsal placement of the male urethral opening (epispadias) and an exposed bladder (exstrophy) - Cloacal Exstrophy: Bladder & portion of the large intestine outside of abdominal wall (MOST SEVERE) Patho: - Failure of the abdominal wall & underlying structures, including the ventral wall of the bladder to fuse in utero Therapeutic/Nursing Management: - Preserve renal function - Attainment of urinary control - Adequate reconstructive repair for acceptable appearance - Prevention of UTIs - Prevention of optimum external genitalia w/ continence and sexual function - Limit trauma to exposed bladder - Monitor I's & O's - Monitor for signs of UTI or other infections Surgical Approaches: - Modern staged repair of exstrophy (MSRE) - Complete primary repair of bladder exstrophy (CPRE)

Drug Reactions of the Skin

Reactions to medications are seen more often in the skin then any other organ - Due to toxicity, individual tolerance or an allergic reaction - IV drugs are more likely to cause a reaction than oral drugs Clinical Manifestations: Can be delayed or immediate - Rashes; exanthematous, urticarial or eczematoid (most common rxns) - Steven-Johnson Syndrome Drug Eruption: - Widespread; appears as an inflammatory response with itching, is sudden in onset and may be associated with constitutional symptoms such as fever, malaise, GI upset, anemia, or liver & kidney damage - Fixed eruption: Recurrent eruption at the same site with each administration of the offending drug Treatment/Nursing Management: - Discontinuing the drug! Stop IV drug immediately & initiate normal saline - Prevention, documentation & assessment - Frequent offenders are Penicillins & Sulfonamides and nurse should be alert to this

Hemophilia

Refers to a group of bleeding disorders resulting from congenital deficiency or dysfunction or absence of specific coagulation proteins or factors Types: - Factor VIII (8) deficiency (Hemophilia A or Classic) most common - Factor IX (11) deficiency (Hemophilia B or Christmas disease) - Von Willebrand Disease (vWD) absence of the von Willebrand factor (vWF) Diagnosis: - History of bleeding episodes - Evidence of X-linked inheritance - Laboratory findings - Factor VIII & Factor IX assays - Partial thromboplastin time (PTT) Clinical Manifestations: - Prolonged bleeding anywhere from or in the body - Hemorrhage from any trauma: Loss of deciduous teeth, circumcision, cuts, epistaxis, injections - Excessive bruising even from a slight injury such as a fall - Subcutaneous & intramuscular (IM) hemorrhages - Hemarthrosis (bleeding into the join cavities), especially in the knees, ankles & elbows - Hematomas: Pain, swelling & limited motion - Spontaneous hematuria Therapeutic/Nursing Management: - DDAVP (Vasopressin) : Increases plasma factor VIII activity (causes 2-4x increase in factor VIII) ONLY WORKS FOR MILDDDDDDDDDDD HEMOPHILIA - Replace missing clotting factors/Transfusions - Corticosteroids given for hematuria, acute hemarthrosis and chronic synovitis - AVOID ASPIRIN & IBUPROFEN (G.I bleeding) - Amicar prevents clot destruction - Physical activity in reasonable limits to strengthen joints & muscles - Family teaching of venipuncture to administer medications - Prophylactic therapy: Infusion of Factor VIII concentrate on a regular basis - PREVENT BLEEDING EPISODES! - Minimize oral bleeding with soft toothbrushes, do NOT use water irrigators, warm water before brushing - Children should wear medical identification bracelets - SubQ route preferred over IM injections to prevent bleeding - RICE: Rest, Ice, Compression & Elevation

Hypoxemia

Refers to an arterial oxygen tension that is less than normal - Identified by a decreased arterial O2 saturation - Deoxygenated hemoglobin at 5 g/dL of blood Clinical Manifestations: - Cyanotic/ Tet spells (usually present when SpO2 is 85% or lower!) Occur most frequently in the first year of life (Tetralogy of Fallot) - Low SpO2 levels - Chronic: Leads to Polycythemia & Clubbing Diagnosis: - PaO2 measurement in an 100% oxygen environment (PaO2 of 100 mmHg or higher suggest lung disease and PaO2 lower than 100 mmHg suggest cardiac disease) - Accurate history, chest radiograph, Echocardiogram Treatment: - Prostaglandin E (vasodilator) - Treatment of hypercyanotic/tet/blue spells (IV insertion for Morphine, Fluids) - Hydration!!! To keep hematocrit & blood viscosity within acceptable limits to reduce risk of CVA - Administration of 100% oxygen for Tet spells - Surgical intervention for cardiac defects Nursing Care Management: - Address parents fears & questions - PREVENT DEHYDRATION! (risk for CVA!) - IVF - Respiratory & Cardiac assessments - Hand hygiene!

Meckel Diverticulum

Remnant of the fetal omphalomesenteric duct, which connects the yolk sac with the primitive midgut during fetal life - Normally obliterated between 5th & 7th week of gestation when placenta replaces yolk sac - 2:1 male to female ratio - Located within 2 feet of the ileocecal valve Clinical Manifestations: - Rectal bleeding caused by ulceration - Bright red/currant jelly like stools or tarry stools - Hypotension (if bleeding is significant) - Abdominal pain/distention, N/V Diagnosis: - Hx & physical exam - Meckel Scan - CBC & Basic metabolic panel Nursing Care Management: - Frequent monitoring of vital signs - Recording I & O's and blood lost in stool - IV fluids & NG tube for decompression & evacuation of gastric secretions (post-op)

Vesicoureteral Reflux (VUR)

Retrograde flow of urine from the bladder into the Upper Urinary Tract - Does NOT cause UTI's but increases the risk that a lower UTI will become Pyelonephritis - When voiding, urine is swept back up into the ureters and bladder, where it becomes a reservoir for bacterial growth until the next void Primary: - Results from congenitally abnormal insertion of ureters into the bladder Secondary: - Occurs as a result of acquired condition Therapeutic/Nursing Management: - Prevent bacteria from reaching the kidneys - Low-dose Abx therapy - Urine culture every 2-3 months or any time a child has a fever - VCUG to assess the status - Surgical intervention if not outgrown

Wounds

Structural or physiologic disruptions of the integument that call for normal or abnormal tissue repair responses Classifications: - Acute: Heal uneventfully within 2-3 weeks - Chronic: Do not heal within expected timeframe & are associated with complications Types: - Abrasions (removal of superficial layer of skin/scrape) - Avulsion (forcible pulling out or extraction of tissue) - Laceration (torn or jagged wound; accidental cut wound) - Incision (division of the skin made with a sharp object; cut) - Penetrating wound (disruption of the skin surface that extends into underlying tissue or into a body cavity) - Puncture ( wound with a relatively small opening compared with the depth)

Intussusception

Telescoping or invagination of one portion of the intestine into another - Most common between children 3 months- 6 years old - More common in males then females, more common in children under 2 - Idiopathic Clinical Manifestations: - Crampy abdominal pain - Inconsolable crying - Drawing up of the knees to chest - Bilious vomiting - Classic Triad: Abdominal Pain, Abdominal Mass (SAUSAGE), Blood Stools - Diarrhea, anorexia, weight loss, vomiting - Lethargy Diagnosis: - Ultrasound: reveals a mass & "Bull's Eye" - Rectal examination Therapeutic/Nursing Management: - Air enema, Hydrostatic (saline) enema - IV fluids, NG decompression - Antibiotics - Hydrostatic Reduction - Fluid volume & electrolyte replacement - Frequent assessment of vital signs (return of bowel sounds)

Tricuspid Atresia

The Tricuspid Valve fails to develop - RIGHT to LEFT blood flow, Cyanotic - No communication from the right atrium to the right ventricle - Defects with decreased pulmonary blood flow Clinical Manifestations: - Cyanosis in newborn period - Tachycardia, Dyspnea

Chronic Renal Failure (CRF)

The diseased kidneys can no longer maintain the normal chemical structure of body fluids under normal conditions - Progresses to a clinical syndrome called Uremia (urea & toxic substances build up) Causes: - Congenital renal & urinary tract malformation - VUR associated with recurrent UTI's - Chronic pyelonephritis - Chronic glomerulonephritis - Lupus Complications: - Retention of waste products (especially BUN & creatinine) - Water & sodium retention - Hyperkalemia - Metabolic Acidosis - Calcium & Phosphorus Disturbances (leads to Renal Osteodystrophy) - Anemia - Growth Disturbance Diagnosis: - History of prior renal disease - S&S of renal dysfunction/impairment Clinical Manifestations: - Loss of normal energy/fatigue - Pallor - Elevated BP - Decreased appetite, less interest in normal activities - Increased or Decreased urinary output - Headache, muscle cramps, nausea - Weigh loss, facial edema, bone or joint pain - Growth retardation - Dryness or itching of the skin - Amenorrhea (common in adolescent girls) - Anorexia, N/V - Bruises, bloody diarrhea, bleeding from lips & mouth - Stomatitis - Uremic frost, Uremic breath odor - Deep respirations - Hypertension - CHF, Pulmonary edema - Seizures, muscle twitching, confusion Therapeutic/Nursing Management: - Promote maximum renal functin - Maintain body fluid & electrolyte balance within safe biochemical limits - Treat systemic complications - Promote as active and normal a life as possible for the child as long as possible - Diet Regulation: Low Protein! - Treat Metabolic Acidosis (Sodium Bicarbonate, Sodium & Potassium Citrate) - Treat Anemia: Epogen (Epoetin Alfa) Injections & Recombinant Human Erythropoietin (rHuEPO) - Control HTN - Treat recurrent infections & seizures - Dietary/Fluid Restrictions; Strict I's & O's; monitor labs - Dialysis

Transposition of the Great Vessels

The pulmonary artery leaves the left ventricle and the aorta exits from the right ventricle with no communication between the systemic and pulmonary circulations - RIGHT TO LEFT, Cyanotic - Usually VSD present (good thing, allows mixing of blood) - Mixed defect Clinical Manifestations: Varies according to the size and shape of the defect - CYANOSIS - Heart Failure Treatment: - Surgical; Swapping of vessels

Total Body Water (TBW)

The total amount of water in the body at a given time - Contains Intracellular Fluid (ICF) & Extracellular Fluid (ECF) - Children more ECF then ICF (can get dehydrated faster) - 45% (in late adolescence) of total body weight - 75% (in term newborns) of total body weight

Acute Glomerulonephritis (AGN)

Type of kidney disease in which the glomeruli the part of the kidney that helps filter waste & fluid from the blood is damaged Causes: - Most are postinfectious or viral - Acute Poststreptococcal Glomerulonephritis (APSGN) Latent period of 10-21 days between infection & onset of manifestations - May be manifestation of a systemic disorder like Systemic Lupus Erythematosus (SLE) & Sickle Cell Disease Diagnosis: - Streptococcal infection - Urinalysis (shows hematuria & proteinuria) - Azotemia, elevated BUN & creatinine levels - Antistreptolysin O (ASO) titer - X-Ray (shows cardiac enlargement & pulmonary congestion or pleural effusion) - Renal biopsy Clinical Manifestations: - Generalized edema due to decreased glomerular filtration (especially in the Periorbital area, Facial edema in the morning, Spreads during the day to involve the lower extremities & the abdomen) - Hypertension due to increased ECF - Oliguria - Hematuria (smoky brown urine, tea or cola colored) - Proteinuria Therapeutic/Nursing Management: - Moderate sodium restriction & fluid restriction may be instituted for children with HTN & edema - Potassium-rich foods are restricted during oliguria - Frequent assessment of vitals, body weight and I's & O's - Daily Weights & Abdominal Girth - Children that develop AKI will have electrolyte imbalances leading to the need for Peritoneal or Hemodialysis - Frequent assessment of BP if they have Hypertension - Regular diet with NO added salt, Low-to Moderate Protein - Antihypertensives & Diuretics

Hemolytic Uremic Syndrome (HUS)

Uncommon, acute renal disease that occurs primarily in infants and small children between 6 months & 5 years old - Most frequent cause of AKI in children - Characterized by Hemolytic Anemia, Acute Renal Failure (ARF) and Thrombocytopenia Cause: - E.Coli Infection! Diagnosis: - Triad of Anemia, ARF & Thrombocytopenia - Increased BUN & creatinine levels - Low hemoglobin & hematocrit & high reticulocyte count Clinical Manifestations: - Vomiting - Irritability, lethargy, pallor - Bruising, petechiae, jaundice, blood diarrhea - Oliguria or anuria - Seizures, stupor or coma - Signs of acute heart failure (rare) Therapeutic/Nursing Management: - Dialysis (Hemo or Peritoneal) - RBC and/or platelet transfusions - Management of chronic disease (AKI)

Hypoplastic Left Heart Syndrome

Underdevelopment of the left side of the heart with significant hypoplasia of the left ventricle including atresia, stenosis or hypoplasia of the ascending aorta and arch - RIGHT TO LEFT, Cyanotic - Mixed defect Clinical Manifestations: - Mild cyanosis - HF until PDA closes - Severe cyanosis & decreased C.O Therapeutic Management: - Prostaglandin E

Burn Injury

Usually attributed to extreme heat sources but may also result from exposure to cold, chemicals, electricity or radiation - Extent of injury described as Total Body Surface Area Burns Related to Age: - Toddlers: Hot-water scalds - Older Children: Flame-related burns - Structural fires occur when children play w/ matches or lighters (majority are males) - Non Accidental burns indicate maltreatment/Child Abuse Depth of Injury: First Degree: Superficial - Epidermis remains intact w/o blisters - Erythema; skin blanches w/ pressure - Painful - Discomfort last for 48-72 hours Second Degree: Partial thickness - Wet, shiny, weeping surface w/ Blisters - Wound blanches w/ pressure - Painful, VERY sensitive to touch, air currents - Heals in <21 days (superficial partial thickness) - Heals >21 days (deep partial thickness) Third Degree: Full thickness - Color variable (deep red, white, black, brown) - Surface dry, thrombosed vessels visible, NO blanching - Insensate (decreased pinprick sensation) - Autografting is Required for healing Fourth Degree: Full thickness + Underlying tissue (muscles, bone) - Color variable - Charring visible in deepest areas, extremity movement limited - Insensate - Amputation of extremities is likely, Autografting is required for healing Patho of Thermal Injuries: - Systemic response involving capillary permeability - Edema, Hypovolemia, Anemia (heat destruction of RBCs) Complications: - Immediate threat of airway compromise - Profound shock - Infection (local & systemic sepsis) - Inhalation injuries, aspiration, pulmonary edema Emergency Treatment: Minor Burns: - Stop the burning process! - Remove burned clothing & jewelry, apply cold water to the burn or hold the burned area under cool running water - DO Not USE ICE - Do not disturb any blisters that formed unless injury is from a chemical substance - Do not apply anything to the burn, cover with a clean cloth if risk of damage or contamination Major Burns: - Stop the burning process! - Smother the fire (flame burns) - Place victim in the horizontal position, roll victim in a blanket or similar objective (avoid covering the head) - Remove burned clothing & jewelry - Assess for adequate airway & breathing!!! If child is not breathing begin mouth-to-mouth resuscitation - Cover the burn with a clean cloth - Keep the victim warm - Begin IV fluids & oxygen therapy as prescribed Therapeutic Management: - FLUID REPLACEMENT! (assess urinary output, vitals especially the heart rate, adequacy of capillary refill and state of sensorium) - High-Protein, High-Calorie diet to avoid protein breakdown! - Antibiotics for infection - Sedation & analgesia is required for the care of the burned child most commonly used are: Fentanyl (sublimaze), morphine, midazolam (Versed), propofol, ketamine and dexmedetomidine - Application of dressings & topical antimicrobial therapy - Excision of Eschar! (Reduce incidence of infection/sepsis) - Debridement (VERY painful & requires analgesia & sedative before) - Hydrotherapy to cleanse burn - Topical Agents for managing the burn: On OPEN burns cover with antibacterial cream 2-3x day, for Semi Occlusive/Occlusive (closed) burns use sterile gauze to apply to the edges of the wound - Temporary Skin Substitutes: Allograft (homograft), Xenograft, Synthetic skin covering - Permanent Skin Coverings: Sheet graft, mesh graft, autograft, Integra/Alloderm (artificial skin) Cultured epithelial autografts (CEAs), ReCell to apply new skin cells Nursing Care Mangement: - Treatment of burn shock & the management of pulmonary status (ABCs!) - Monitor vital signs, urine output, fluid infusion and respiratory parameters - Constant observation of complications - After patient's condition is stabilized, prevent wound infections, closing the burn as soon as possible - Comfort & Pain management! - Care of the burn wound (Use Atraumatic Care!) - Maintain body temperature - Check pulses in affected extremities (COMPARTMENT SYNDROME 6 P's!) Long-Term Care: - Begins once wound coverage has been achieved - Prevention/management of Contractures! - Physical/occupation therapy - Facilitate adaptation of child & family - Prevention of future burn injuries

Cat Scratch Disease

Usually follows the scratch or bite of an animal (a cat or kitten in 99% of cases) Cause: - Bartonella henselae bacteria Clinical Manifestations: - Painless, nonpruritic erythematous papule at the site of inoculation followed by regional lymphadenitis - Lymph nodes involved usually axillary, epitrochlear, cervical, submandibular, inguinal and preauricular Complications: - Encephalitis - Hepatitis - Parinaud oculoglandular syndrome Diagnosis: - History of contact w/ cat or kitten - Presence of regional lymphadenopathy for several days - Serologic identification of the causative organism by indirect fluorescent antibody assay or polymerase chain reaction test Treatment: - Primarily supportive - 5-day course of oral Azithromycin Nursing Care Mangement: - Prevention & supportive care

Hypospadias

Ventral placement of the male urethral opening - Common congenital anomaly - Cryptorchidism is present in 10% of infants with this - Surgical correction usually warranted Therapeutic/Nursing Management: - Prepare patients for procedure - Surgical correction (around 6-12 months) - Neonatal circumcision should be avoided - Prevent infection - Urinary diversion (catheter & drainage bag)

Peritoneal Dialysis

Wherein the abdominal cavity acts as a semipermeable membrane through which water and solutes of small molecular size move through osmosis and diffusion according to their respective concentrations on either side of the membrane - Preferred method of dialysis for Children - Can be managed at home in some cases - Warmed solution enters the peritoneal cavity and remains for a time before removal

Diaper Dermatitis

inflammatory reaction of the skin in the area covered by a diaper Patho: Caused by prolonged & repetitive contact with the irritant (urine, feces, soaps, detergents, ointments & friction) - Detergents inadequately rinsed from clothing - Chemical irritation (especially from diaper wipes) Clinical Manifestations: - Primarily on convex surfaces or in skin folds (buttocks, inner thighs, mons pubis & scrotum) - Candida albicans can cause inflammation & maculopapular rash w/ satellite lesions that may cross the inguinal folds Nursing Care Management: 3 Factors that cause Dermatitis: Wetness, pH & fecal irritants - Changing the diaper as soon as it becomes wet! - Use super absorbent disposable paper diapers - Expose healthy or only slightly irritated to air not heat, to dry completely - Apply ointment such as zinc oxide or petrolatum to protect skin - Avoid over washing the skin especially with perfumed soaps or commercial wipes - Gently wipe stool from skin using a soft cloth or warm water - Use disposable diaper wipes that are detergent & alcohol free

Normal Urinalysis

pH: 4.8 to 7.8 (average: 6) Specific Gravity: 1.001 to 1.030 Protein: Less than 20 mg/dL Urobilinogen: Up to 1 mg/dL Should NOT Show: - Glucose - Ketones - Hemoglobin (HgB) - WBC's - RBC's - Casts - Nitrites